What is Lupoid Hepatitis?

Lupoid hepatitis, more commonly known as auto-immune hepatitis (AIH), is a disease in which the immune system attacks the cells of the liver and causes inflammation. The term “lupoid” refers to its previous association with those who had the autoimmune disease known as systemic lupus erythematosus (SLE). SLE patients may still have lupoid hepatitis, but as many more cases were found to occur with other autoimmune disorders, the term lupoid hepatitis fell out of favor. In some cases, it can progress and cause lesions and scarring of the liver, a condition known as cirrhosis. It is most common in women, and is considered a chronic disease.

There are two primary classes of lupoid hepatitis: Type 1 and Type 2. Type 1 is the more common one, and it is typically seen in young patients between 10-20 years of age, and adults 45-70 years of age. A large percentage of those with Type 1 also have another autoimmune disease, such as Graves’ disease or Type 1 diabetes. Type 2 is less common and occurs in children, mostly female, from ages 2-14. It is more severe, and it more often progresses to cirrhosis of the liver than Type 1.

The symptoms of lupoid hepatitis can vary, depending on the severity and stage of the disease. People in the earlier stages very often have few symptoms. When symptoms do occur, fatigue is usually the first sign. Other symptoms include abdominal discomfort, itching, and joint pain. Jaundice, a yellowing of the skin and whites of the eyes, can also be seen.

In more progressed cases, nausea and vomiting, along with an enlarged liver, can be symptoms. Blood vessels, called angiomas, will sometimes appear on the abdominal area of the skin. Many of the symptoms can resemble viral hepatitis, so it is important to check for any type of viral infection signs.

Diagnosis for lupoid hepatitis is done either through blood work, which also helps rule out other forms of hepatitis, or liver biopsy. In type 1 lupoid hepatatis, antinuclear antibodies (ANA) and smooth muscle antibodies (SMA) are present, either separately or together; in type 2, Anti-klm (AKLM), antibodies to liver and kidney microsomes, are present.

The most common form of treatment in early and middle stages of the disease is prednisone, a corticosteroid that suppresses the immune system and reduces inflammation. Since prednisone has side effects, dosage is carefully monitored and gradually tapered down to a lower dose. Other drugs that suppress the immune system are also used. In more severe cases, a liver transplant will be performed.

The prognosis for lupoid hepatitis depends on several factors, especially if another disease is involved, but is generally good. It often has to be treated for at least 6 months for the liver blood test results to return to normal, although it can take longer. Sometimes it needs to be treated off and on for a lifetime. In very mild cases, there may be no treatment prescribed.